Diagnosis of berry syndrome in an infant by two-dimensional and color Doppler echocardiography

Abstract

A 6-month-old male infant with coexistent type B interrupted aortic arch, distal aortopulmonary window, and anomalous origin of the right pulmonary artery arising from the ascending aorta was diagnosed by two-dimensional echocardiography, color Doppler, and cardiac catheterization. Review of the available literature reveals this patient to be the fourteenth reported case of this unusual association of cardiovascular defects.

A Case of Berry Syndrome Type 2B

“…In Berry syndrome, no other cardiac or visceral congenital malformation is usually present 1–7 . Correct preoperative diagnosis and detailed anatomic depiction of each component are important in preoperative surgical planning 1–7 . We report a case in which each component of this rare association was defined precisely with computed tomography imaging.…”

“…Subtypes of this syndrome have been described based on the level and type of the aortopulmonary communication, which may be proximal from the main pulmonary artery with normal origins of the right and left pulmonary arteries (Berry syndrome type 1), distal with origin of the right pulmonary artery from the aortopulmonary communication (Berry syndrome type 2A), distal with origin of the right pulmonary artery entirely from the aorta (Berry syndrome type 2B), or with multiple aortopulmonary communications (Berry syndrome type 3). In all of the Berry syndrome subtypes, there is also a patent ductus arteriosus and some degree of aortic coarctation, ranging from a short, stenotic/hypoplastic segment, to interruption/agenesis of the distal portion of the arch 1–7 . In Berry syndrome, no other cardiac or visceral congenital malformation is usually present 1–7 .…”

“…In all of the Berry syndrome subtypes, there is also a patent ductus arteriosus and some degree of aortic coarctation, ranging from a short, stenotic/hypoplastic segment, to interruption/agenesis of the distal portion of the arch 1–7 . In Berry syndrome, no other cardiac or visceral congenital malformation is usually present 1–7 . Correct preoperative diagnosis and detailed anatomic depiction of each component are important in preoperative surgical planning 1–7 .…”